Clinical, neuroimaging, and behavioural risk factors for neurocognitive impairment in Chinese patients with haemophilia: A multicentre study.

BACKGROUND: Few studies have evaluated the impact of subclinical microstructural changes and psychosocial factors on cognitive function in patients with haemophilia. OBJECTIVES: To determine the prevalence and characteristics of cognitive impairment in patients with haemophilia, and identify associated risk factors. METHODS: We recruited haemophilia A or B patients who were aged ≥10 years old from three public hospitals in Hong Kong. A neurocognitive battery was administered to evaluate their attention, memory, processing speed and cognitive flexibility performances. They also underwent magnetic resonance imaging to identify cerebral microbleeds. Validated self-reported questionnaires were administered to assess their mental health status and adherence to prophylactic treatment. General linear modelling was used to investigate the association of neurocognitive outcomes with risks factors, adjusting for age and education attainment. RESULTS: Forty-two patients were recruited (median age 32.0 years; 78.6% haemophilia A; 80.9% moderate-to-severe disease). Six patients (14.3%) had developed cerebral microbleeds. A subgroup of patients demonstrated impairments in cognitive flexibility (30.9%) and motor processing speed (26.2%). Hemarthrosis in the previous year was associated with worse attention (Estimate = 7.62, 95% CI: 1.92-15.33; p = .049) and cognitive flexibility (Estimate = 8.64, 95% CI: 2.52-13.29; p = .043). Depressive (Estimate = 0.22, 95% CI: 0.10-0.55; p = .023) and anxiety (Estimate = 0.26, 95% CI: 0.19-0.41; p = .0069) symptoms were associated with inattentiveness. Among patients receiving prophylactic treatment (71.4%), medication adherence was positively correlated with cognitive flexibility (p = .037). CONCLUSION: A substantial proportion of patients with haemophilia demonstrated cognitive impairment, particularly higher-order thinking skills. Screening for cognitive deficits should be incorporated into routine care. Future studies should evaluate the association of neurocognitive outcomes with occupational/vocational outcomes.

Telehealth Interventions for Improving Self-Management in Patients With Hemophilia: Scoping Review of Clinical Studies.

BACKGROUND: The introduction of home therapy for hemophilia has empowered patients and their families to manage the disease more independently. However, self-management of hemophilia is demanding and complex. The uses of innovative interventions delivered by telehealth routes such as social media and Web-based and mobile apps, may help monitor bleeding events and promote the appropriate use of clotting factors among patients with hemophilia. OBJECTIVE: This scoping review aims to summarize the literature evaluating the effectiveness of telehealth interventions for improving health outcomes in patients with hemophilia and provides direction for future research. METHODS: A search was conducted in Ovid MEDLINE, EMBASE, and PubMed databases for studies that (1) focused on patients with hemophilia A or B; (2) tested the use of remote telehealth interventions via the internet, wireless, satellite, telephone, and mobile phone media on patients and caregivers; and (3) reported on at least one of the following patient-/caregiver-focused outcomes related to empowering patients/caregivers to be active decision makers in the emotional, social, and medical management of the illness: quality of life, monitoring of bleeding episodes, joint damage or other measures of functional status, medication adherence, and patients' knowledge. Implementation outcomes (user metrics, cost saving, and accuracy of electronic records) were also evaluated. Reviews, commentaries, and case reports comprising ≤10 cases were excluded. RESULTS: Sixteen articles fulfilled the inclusion criteria. The majority of the interventions (10/16, 62%) evaluated both implementation outcomes and patient-/caregiver-focused outcomes. User performance and accuracy and comprehensiveness of electronic records were also measured in most studies (4/16, 87%). The components of the interventions were rather homogenous and typically involved electronic logging and reminders for prophylactic infusions, reporting of spontaneous and traumatic bleeding events, monitoring of infusion product usage and home inventory, and real-time communication with health care professionals and hemophilia clinics. Telemedicine-supported education and information interventions seemed to be particularly effective among adolescent and young adult patients. Although the patients reported improvements in their health-related quality of life and perception of illness, telemonitoring devices did not appear to have a significant effect on quantifiable health outcomes such as joint health. Longitudinal studies seemed to suggest that the response and adherence rates to recording decreased over time. CONCLUSIONS: Preliminary evidence from this review suggests that telehealth-delivered interventions could feasibly improve patients' adherence to medication use and promote independence in disease management. Given the complexity and resources involved in developing a mature and established system, support from a dedicated network of hemophilia specialists and data managers will be required to maintain the technology, improve adherence to prophylactic treatment and recording, and validate the electronic data locally.

Treatment Adherence and Health-Related Quality of Life in Patients with Hemophilia in Hong Kong.

Background: This study aims to identify factors affecting health-related quality of life (HRQoL) in Chinese patients with hemophilia in Hong Kong, and to examine the association between treatment adherence and HRQoL outcomes. Methods: Patients with hemophilia A or B from a non-governmental organization reported their HRQoL and treatment adherence to prophylactic therapy using validated tools. Univariate tests and multivariable regression analysis were used to compare differences in outcomes across clinically relevant subgroups. Results: Fifty-six patients were recruited (mean age 30.4 [17.4] years; majority hemophilia A: 75%; moderate-to-severe severity: 88%). Patients who received prophylactic treatment reported fewer work/school problems (25.8 [18.9] versus 51.5 [26.3]; p = 0.001) than those who received on-demand therapy. The multivariable model showed that older age (B = 0.42, 95% CI = 0.093−0.75) and living in public housing (B = 10.24, 95% CI = 0.70−19.77) were associated with worse HRQoL. Older age was associated with treatment non-adherence (r = 0.66, p < 0.0001). Patients with poor adherence tended to report worse functioning in sports/leisure (r = 0.31, p = 0.033). Conclusions: Our results suggest that patients who were older, had lower education attainment and received on-demand treatment had poorer perception of their health. Improving adherence may lead to better HRQoL. Future work includes evaluating the occupational needs prospectively in this population.

Technology Acceptance Among Patients With Hemophilia in Hong Kong and Their Expectations of a Mobile Health App to Promote Self-management: Survey Study.

BACKGROUND: The lifelong management of hemophilia is demanding and complex. In July 2019, we published a review in the Journal of Medical Internet Research, summarizing telehealth interventions that facilitate monitoring of bleeding events and promoting the appropriate use of clotting factors among patients with hemophilia. This work has led to the development of a community program that aims to harness technology to promote self-management among patients with hemophilia in Hong Kong. OBJECTIVE: Before the inception of this program, we conducted a cross-sectional survey to evaluate the patients' level of technology acceptance and identify their expectations of the use of mobile technology for self-management of hemophilia. METHODS: In total, 56 participants (75% adult patients and 25% parents of pediatric patients; 87.5% with moderate to severe disease) were recruited from a local nongovernmental organization that serves patients with hemophilia. They rated their perceived confidence and acceptance in using the new mobile technology (score 1 to 5 for each item, with a higher score indicating better acceptance) using a structured questionnaire (adapted from the Technology Acceptance Model). They also identified the top features that they perceived to be the most important components of a mobile app for the self-management of hemophilia. The Mann-Whitney U test was used to compare technology acceptance scores across subgroups of different clinical and socioeconomic characteristics. RESULTS: In general, the participants considered themselves skilled in using mobile apps (mean 4.3, 95% CI 4.1-4.5). They were willing to learn to use the new mobile app to organize their bleeding records (mean 4.0, 95% CI 3.7-4.3) and to manage their health (mean 4.2, 95% CI 4.1-4.5). Participants who lived in public housing (a surrogate marker for lower socioeconomic status in Hong Kong) reported lower technology acceptance than those who lived in private housing (P=.04). The most important features identified by the participants concerned documenting of infusion logs (n=49, 87.5%), bleeding events (n=48, 85.7%), and the secure delivery of the bleeding information to health care professionals (n=40, 71.4%). CONCLUSIONS: It is encouraging to infer that patients with hemophilia in Hong Kong are receptive to the use of mobile health technology. The findings of this survey are applicable in designing the key features of a patient-centered, multimodal program harnessing mobile technology to promote self-management among patients with hemophilia. Future studies should evaluate participants' acceptability and perceived usability of the mobile app via user metrics and assess clinical and humanistic outcomes of this program.